The gentle giant – A case sacrococcygeal chordoma

Introduction

Spinal cord tumors occur within or around the spinal cord. Spinal cord tumors can be intramedullary (i.e, inside the spinal cord, ex., ependymomas, astrocytomas), intradural extra medullary (Tumors arising within the dura but outside of the spinal cord, ex., meningiomas), or extradural (arising in the vertebral bodies, ex, chordomas). Chordomas are primarily rare bone tumors that arise from notochord remnants and generally manifest in middle-aged males, mainly involving the sacrococcygeal region. 50% to 60% of cases of chordomas involve the sacrococcygeal region, while 30% to 35% involve the clivus or spheno-occipital region [1]. Seldom are vertebral bodies involved. Chordomas are called ‘gentle giants’ due to their slow-growing, indolent nature despite their potential for extensive local invasion and tissue destruction. While they are locally aggressive, they rarely metastasize in early stages, which contributes to the misconception that they are less harmful. However, due to their ability to encase critical neurovascular structures, they can be difficult to resect completely, leading to high recurrence rates [2]. The reason behind the development of chordomas in specific notochordal residual cells is unknown. Chordomas are the most prevalent primary malignant sacral tumors, making up around 2%–4% of all initial malignant bone tumors [3] [4] [5]. The sacrococcygeal region has rather large tumor sizes upon presentation, which can be explained by the tumor’s slow growth [6]. Depending on their anatomical sites, chordomas can present with a wide range of symptoms, most commonly in the spine or at the lower part of the skull.

Publication History

Received: 06 December 2024

Accepted after revision: 05 May 2025

Article published online:
17 June 2025

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